Purpose: To report a case of an eyelid neurothekeoma, a rare peripheral nerve sheath tumor. Methods: Case report. Results: An excisional biopsy, performed on a lesion removed from the upper eyelid of a 76-year-old woman, revealed the clinical and histopathologic features of a neurothekeoma, a tumor consisting of multiple collections of spindle cells in a myxomatous background. Immunohistochemical characterization showed positivity for NK1/C3, neuron-specific enolase, and alcian blue. Conclusion: Neurothekeoma palpebrae should be considered in the differential diagnosis of solitary nodules of the eyelids.